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Obstructive sleep apnea in late-onset Pompe disease treated by enzyme replacement therapy
Neuromuscular Disorders ( IF 2.7 ) Pub Date : 2020-04-01 , DOI: 10.1016/j.nmd.2020.02.004
Raj Bhui 1 , Andrew R Spector 1
Affiliation  

Obstructive sleep apnea is a common complication of Pompe disease. Treatment for obstructive sleep apnea in patients with Pompe disease is similar to treatment in the general population, typically involving positive airway pressure therapy. We present a case in which a patient with late-onset Pompe disease was able to discontinue positive airway pressure therapy after treatment with enzyme replacement therapy for his Pompe disease. It is likely that an improvement in muscle tone from the enzyme replacement therapy was sufficient to eliminate his obstructive sleep apnea. Pharmacological therapies for obstructive sleep apnea are lacking but could apply to certain populations, such as Pompe disease.

中文翻译:

酶替代疗法治疗迟发性庞贝病的阻塞性睡眠呼吸暂停

阻塞性睡眠呼吸暂停是庞贝病的常见并发症。庞贝病患者阻塞性睡眠呼吸暂停的治疗与一般人群的治疗相似,通常涉及气道正压治疗。我们介绍了一个案例,其中一名迟发性庞贝病患者在接受庞贝病酶替代疗法治疗后能够停止气道正压治疗。酶替代疗法对肌肉张力的改善很可能足以消除他的阻塞性睡眠呼吸暂停。目前缺乏针对阻塞性睡眠呼吸暂停的药物疗法,但可能适用于某些人群,例如庞贝病。
更新日期:2020-04-01
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